II. Associated Conditions

  1. Cancer (esp. Small Cell Lung Cancer) in 50% of cases

III. Pathophysiology

  1. Similar to Myasthenia Gravis (in that acetylcholine is not functional at Synapse)
  2. Calcium channel antibodies block acetylcholine release from presynaptic terminals

IV. Symptoms

  1. Proximal Muscle Weakness and Fatigue (better with use)
    1. Thighs and hips are most affected (contrast with initial bulbar symptoms of myasthenia)

V. Management

  1. Plasmaphoresis
  2. Tumor resection
  3. Avoid provocative agents
    1. Neuromuscular blocking agents
    2. Aminoglycosides
    3. Intravenous Contrast dye

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Ontology: Lambert-Eaton Myasthenic Syndrome (C0022972)

Definition (MSHCZE) Paraneoplastický stav provázející malobuněčný bronchogenní karcinom plic připomínající klinicky myastenii (pseudomyastenie). Paraneoplastický syndrom charakterizovaný nervosvalovými poruchami a vegetativní dysfunkcí, svalová slabost, hyporeflexie, ptóza, xerostomie, ortostatická hypotenze, trávicí obtíže aj.. Podstatou je vznik autoprotilátek proti iontovým kanálům. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A rare autoimmune presynaptic disorder characterized by impairment of the impulse transmission at the neuromuscular junction. It affects predominantly the proximal muscles of the lower extremities, resulting in muscle weakness and fatigability. It has been associated with small cell lung carcinoma.
Definition (MSH) An autoimmune disease characterized by weakness and fatigability of proximal muscles, particularly of the pelvic girdle, lower extremities, trunk, and shoulder girdle. There is relative sparing of extraocular and bulbar muscles. CARCINOMA, SMALL CELL of the lung is a frequently associated condition, although other malignancies and autoimmune diseases may be associated. Muscular weakness results from impaired impulse transmission at the NEUROMUSCULAR JUNCTION. Presynaptic calcium channel dysfunction leads to a reduced amount of acetylcholine being released in response to stimulation of the nerve. (From Adams et al., Principles of Neurology, 6th ed, pp 1471)
Concepts Disease or Syndrome (T047)
MSH D015624
ICD9 358.3
ICD10 G73.1 , G70.80
SnomedCT 230688006, 56989000
English Eaton Lambert Myasthenic Syndrome, Eaton Lambert Syndrome, Eaton-Lambert Myasthenic Syndrome, Eaton-Lambert Syndrome, Lambert Eaton Syndrome, Lambert-Eaton Myasthenic Syndrome, Lambert-Eaton Syndrome, Myasthenic Syndrome, Eaton-Lambert, Myasthenic Syndrome, Lambert-Eaton, Syndrome, Eaton-Lambert Myasthenic, Syndrome, Lambert-Eaton, Syndrome, Lambert-Eaton Myasthenic, Syndrome, Eaton-Lambert, Eaton Lamb myasthenic syndr, Eaton Lambert myasthenic syndrome, LEMS - Lambrt-Eatn myasth synd, Lambert-Eaton myasthenic syndr, MYOPATHIC MYASTHENIC SYNDROME OF LAMBERT EATON, MYASTHENIC MYOPATHIC SYNDROME LAMBERT EATON, Myasthenic Syndrome, Lambert Eaton, Eaton-Lambert syndrome (diagnosis), Eaton-Lambert Myasthenic-Myopathic Syndrome, Eaton-Lambert Myopathic-Myasthenic Syndrome, Lambert-Eaton Myasthenic-Myopathic Syndrome, Lambert-Eaton Myopathic-Myasthenic Syndrome, Myasthenic-Myopathic Syndrome of Eaton-Lambert, Myasthenic-Myopathic Syndrome of Lambert-Eaton, Myopathic-Myasthenic Syndrome of Eaton-Lambert, Myopathic-Myasthenic Syndrome of Lambert-Eaton, Lambert-Eaton Myasthenic Syndrome [Disease/Finding], eaton lambert syndrome, myasthenic syndrome, eaton-lambert syndrome, lambert-eaton syndrome, lambert eaton syndrome, myasthenic syndromes, Eaton Lambert syndrome, Lambert Eaton myasthenic syndrome, Lambert Eaton syndrome, myasthenic syndrome of Lambert Eaton, Lambert-Eaton Myasthenic-Myopathic Syndromes, Myasthenic Myopathic Syndrome of Eaton Lambert, Myasthenic Myopathic Syndrome of Lambert Eaton, Myopathic Myasthenic Syndrome of Eaton Lambert, Lambert-Eaton syndrome, Lambert-Eaton Myopathic-Myasthenic Syndromes, Eaton-Lambert Myopathic-Myasthenic Syndromes, Lambert-Eaton syndrome NOS, Eaton Lambert myasthenic syndrome (disorder), Eaton-Lambert syndrome, LEMS - Lambert-Eaton myasthenic syndrome, Lambert-Eaton myasthenic syndrome, Myasthenic syndrome, Eaton-Lambert syndrome (disorder), Lambert-Eaton (etiology), Lambert-Eaton (manifestation), Eaton-Lambert (etiology), Eaton-Lambert (manifestation), Lambert Eaton Myasthenic Syndrome
French Syndrome myasthénique d'Eaton-Lambert, Syndrome de Lambert-Eaton, Syndrome myasthénique de Lambert-Eaton
Swedish Lambert-Eaton myastent syndrom
Czech Lambertův-Eatonův myastenický syndrom, Lambert-Eatonův syndrom, Lambert-Eatonův myastenický syndrom, Eatonův-Lambertův syndrom, LEMS, Lambertův-Eatonův-Rookeho syndrom, Lambertův-Eatonův syndrom
Spanish Síndrome miasténico de Eaton-Lambert, síndrome de Eaton - Lambert (trastorno), síndrome de Eaton - Lambert, síndrome miasténico, Síndrome de Eaton-Lambert, Síndrome de Lambert-Eaton Miasténico, Síndrome Miasténico de Lambert-Eaton
Dutch myastheen syndroom van Lambert-Eaton, Eaton-Lambert-syndroom, Lambert-Eaton-syndroom, Myasthenisch syndroom, Eaton-Lambert, Syndroom van Eaton-Lambert
Portuguese Síndrome miasténico de Eaton-Lambert, Síndrome de Eaton-Lambert, Síndrome Miastênica de Lambert-Eaton
German Lambert-Eaton Syndrom, Lambert-Eaton-Syndrom, Eaton-Lambert-Syndrom, Lambert-Eaton-Myasthenie-Syndrom, Myasthenie-Syndrom, Lambert-Eaton-
Japanese ランバート・イートン筋無力症候群, イートンランバートショウコウグン, イートン・ランバート症候群, ランバートイートンキンムリョクショウコウグン, イートン・ランバート症候群, ランバート・イートン症候群, Eaton-Lambert症候群, Lambert-Eaton症候群, Lambert-Eaton筋無力症候群, Lambert-Eaton筋無力症症候群, ランバート-イートン筋無力症症候群, 筋無力症症候群, ランバート-イートン症候群
Finnish Myasteeninen oireyhtymä
Italian Sindrome di Eaton-Lambert, Sindrome miastenica di Eaton-Lambert, Sindrome miopatico miastenica di Eaton-Lambert, Sindrome miopatico miastenica di Lambert-Eaton, Sindrome di Lambert-Eaton, Sindrome miastenica di Lambert-Eaton
Korean 이튼-람베르트 증후군
Polish Zespół miasteniczny Lamberta-Eatona, Zespół Lamberta-Eatona
Hungarian Eaton-Lambert-syndroma, Lambert-Eaton myastheniás syndroma
Norwegian Lambert-Eaton myastenisk syndrom, Eaton-Lambert syndrom